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Maxillary angiosarcoma
lilt. J. Oral Maxillofac. Surg , 1986: 15: 357-360 (Key wo rds: sarcoma ; angiosarcoma ; malignancy. oral; surgery. oral and maxillofacial)
Maxillary angiosarcoma NICHOLAS ZACHARIADES AND PANAYIOTA ECONOMOPOULOU Oral and Maxillofacial Department, "Apostle Paul's" Accidents Hospital , Kiflssia, Athens, Greece and Department of Oral Pathology, Dental School, National University, Ath ens, Greece
ABSTRACT - A malignant tumor of mesenchym al origin, angiosarcoma, is a rare entity deriving from the endothelium of the blood vessels, that very infrequ ently is encountered in the jaws. 46 such cases have only been reported.
(Accepted for publication Zil March 1985)
Angiosarcoma is a very rare tumor considering the abundance of blood vessels in the human body. It is known to develop as a primary tumor or from irradiated benign hemangioma 1.6.16,21,23,27,31 . It usually affects younger people'. Although the cutaneous type is usually encountered in the face and scalp, the intraosseous type is seldom reported in the jaws l ,6,26,36. Because of its rarity, it is seldom included in difTerential diagnosis and almost never diagnosed clinically. Pyogenic granuloma is the more likely diagnosis. The central type will appear as a radiolucent lesion in radiographic examination, which in some cases may simulate a benign lytic lesion ' ,23,26,31. Even microscopically, it is not always easy to distinguish between the benign and the malignant forms , except for the atypical endothelial cells that characterize the latter, Yet it is still difficull to distinguish between hemangioendotheliosarcorna and hemangiopericytosarcoma, and in more anaplastic forms, between these tumors and leiomyosarcoma or fibrosarco-
ma S,22,24,27,2 8,33,36,39. The tumor spreads by local invasion and metastasizes mainly by the bloodstream. The best treatment consists of wide surgical resection followed by chemotherapy. Prognosis is poor; few patients survive longer than 2 years. Pulmonary metastasis is the main cause of dea th 1.14,21,23,26,28,31,36,39 .
Report of a case A 68-year old woman was admitted to the hospital because of a painless swelling of the left infraorbital region (Fig. I) of 2 months duration. The swelling was associated with infraorbital nerve hypesthesia and could also be palpated . intraorally at the corresponding muccobu ccal fold. No regional lymph nod es were palpable. Radiographic examination (Fig. 2) revealed that a diffuse rad iolucency occupied the area between the upper left central incisor and the canine, also extending towards the premolars. The left lateral incisor had been extr acted and the left central incisor and canine had had root canal therapies performed a month previously. (There was no evidence of caries in the central incisor.) The den-
358
ZACHARIADES AND ECONOMOPOULOU
tal work was carried out because these teeth were painful and the swelling of the -area was considered to be an abscess originating from them. Antibiotics had been prescribed, but the swelling did not subside. Suspecting a malignancy, it was decided to admit the patient for a detailed clinical and laboratory examination, which proved within nonnal limits. 2 days later, under local . anesth esia, a mucoperiosteal nap was raised and a biopsy was performed. The pathologist described sheets of mesenchymal cells that resembled endothelial cells and newly fonned fibroblasts. There were a number of mitoses as well as pleomorphism of cells. Vascular channels were also seen in the specimen (Figs. 3, 4). The diagnosis was angiosarcoma; the patient, however, discharged herself following diagnosis and has not been seen since. Fig. J. Appearance of the patient upon admisWe have tried to locate her and we learned that sion. Note the swelling of the left infraorbital she visited a specialized clinic abroad, where a region. new biopsy was taken, which verified the original
"..'
.. . 4'
'.
.... _ ;. ~
• •
, ,, ~
" ..
'-
Fig. 2. Radiograph of the involved area showing a diffuse radiolucency extending between the upper left central incisor and the premolars of th e same side.
. .
-
Fig . 3. Photomicrograph showing atypical capil-
laries lined with anaplastic endothelial cells. The lumina of most of the capillaries are filled with neoplastic cells. (H.&E. x 160.)
359
MAXILLARY ANGIOSARCOMA 9 8 7 1Il
6
~ 5
:34 3 2
I
I
I
1 ............~~~~~.....~.......~.....~~_ 10 20 30 40 50 60 70
age Fig. 5. Age distribution of the cases presented, when indicated in the reports. Fig. 4. Irregular vascular channels lined with proliferating endothelial cells that ar e pleomorphic and show large hyperchromatic nuclei. (H.&E. x 63.)
dia gnosis of angiosarcoma. (We were able to sec the pathologist's report but the micro scopic slides were not available.) The tumor was cons idered inopera ble and chemotherapy was adm inistered . The patient stayed abroad for approximately 6 months. There was some remi ssion of her symptom s for up to about a year, after which she returned home . At that time she started to develop dyspnea, back pain and head aches. She visited the same clinic abroad, where recurrence of the tumor was verified, associated with wide spread metastasis that involved both lungs, the bra in, the liver and the lumbar spinal column. No further biopsies were taken this time and according to her relatives chemotherapy and irradiation were administered. The patient stayed abroad for a month and was brought back home to die, 15 days later, approximately 2 years after the initial biopsy was taken by us.
Discussion Angiosarcoma is a very rare neoplasm; only
46 cases have so far been reported in the oral cavity and the surrounding tissue sl-l.7-21.2+-26,29-J2,.l-l-39. The age of the patients, when recorded, varied, the youngest being I-day old' and the oldest (current report) 68 (Fig.5). The male/female ratio is almost 1:2 and the mandible and the maxilla are affected with approximately the same frequency. There is no difference between
the right and the left side of the face. The radiographic picture, when published in reports, is always osteolytic. The best treatment appears to consist of wide surgical removal of the tumor regardless of the combination of irradiation. The 2-year survival rate is 50% and the 5-year rate is only 25%. We had the opportunity to encounter another such rare case in OUr Institution".
References I. ALBRUlT, C. R .: Angiosarcoma of the gingiva: report of case. J. Oral Surg, 1970: 28: 913-917. 2. ARENA, S.: Somatic tissue tumors in the head and neck. Trans. Acad. Ophthalmol. Otolarygal. 1962: 15: 100. 3. BERGER, A.: Hemangiosarcoma of the mandible (metastatic?) Ann. Dent. 1942: 1: 15-20. 4. BLAKE, H. , BLAKE, F. S. & PATERSON, N. J.: Angiosarcoma. Oral Surg, 1956: 9: 821-825. 5. BLOOM, W. B. & FAWCETI, D. \V.: Textbook ofhistology, W. B. Sawnders Company, Philadelphia 1969, pp. 358-361. 6. Buxnsxs , W. D. & BRIGIITON, C. T.: Malignant hemangioendothelioma of bone. J . Bone Joint Surg , 1965: 47A: 762-772. 7. CALHOUN, J. J .: Malignant hemangioendothelioma (ang iosarcoma). Oral Surg. 1969: 27: 156-160. 8. CARR, M. W.: Congenital bilateral hem an gioendothelioma. J. Oral Surg, 1948: 6: 341-350. 9. CHEYNE, V.D. & SILBERSTEIN, H. E.: Hemangio-endothelioma. Am. J. Orthod. 1942: 28: 703-721.
360
ZACHARIADES AND ECONOMOPOULOU
10. DELARUE, J.: Observations de tumeurs reticulaires malignes adebut maxillaire en generalisant rapidement. Rev. Stomatal. Chir. Maxillofac. 1957: 58: 603-610. 11. ENDOKIMOV, A. I., GORBUSCIiINA, P. M. & VOROBEZ, I. I.: Malignant hemangioendothelioma of the mandible. Stomatologia 1963: 42: 43--47. 12: FARR, H. W., GARANDANG, C. M. & Huvos, A G.: Malignant vascular tumors of the head and neck. Am. J. Surg. 1970: 120: 501-504. 13. GANDIII, P. K.: Hemangiosarcoma (malignant hemangioendothelioma) of the mandible in a child. Oral Surg. 1966: 22: 359-362. 14. GIRARD, C., WAINE, C. J. & GRAHAM, J. H.: Cutaneous angiosarcoma. Cancer 1970: 23: 868-883. 15. GONDAERT, M.: Angiosarcome du maxillaire superieur (Hemangio-endotheliorne malin). Rev. Stomato-Odontol. 1968: 15: 159-172. 16. HARTMAN, W. H. & STEWART, F. W.: Hemangioendothelioma of bone; unusual tumor characterized by indolent course. Cancer 1962: 15: 846-854. 17. HENNY, F. A.: Angioma of the maxilla in a 3-month old infant: report of case. J. Oral Surg. 1949: 7: 250-252. 18. KRISIINA, G., TIAGI, G. K. & TIAGI, P. S.: Surg, Hemangioendotheliosarcoma. Int. 1968: 50: 377-380. 19. KUPCIIIK, B.:M. & FORLOv, P.: Angiosarcoma of the tongue. Stomatologiia 1971: 50: 91-93. 20. LEGG, Q. J. & FITCH,W M.: Hemangioendothelioma: review of the literature with a report of two cases. South Surg, 1950: 16: 803-811. 21. NALDlcK, R. A.: Angiosarcoma of the mandible. Plast. Reconstr. Surg. 1969: 43: 92-95. 22. ORLlAN, A. I.: Benign hemangiopericytoma of the tongue. J. Oral Surg. 1973: 31: 936-938. 23. OTIS, J.: Hemangioendothelioma of bone. Surg. Gynecol. Obstet. 1969: 127: 295-305. 24. PIIILlPS, H., BROWN, A. & BALL, M.: Hemangioendothelioma: report of case. J. Oral Surg. 1969: 27: 286-288. 25. PINDBORG, J. J. & PIIILlPSEN, H. P.: Malignant angioblastoma of bone. Acta Pathol. Microbial. Scand. 1960: 49: 408--416. 26. QmNN, J. H., MCCONNELL, H. A. & LEONARD, G. L.: Multifocal angiosarcoma of the
27.
28. 29.
30.
31.
32.
33.
34.
35.
36.
37. 38.
39.
gingiva: report of case. J. Oral Surg. 1970: 28: 215-217. RAMSEY, H. J.: Fine structure of hernangiopericytoma and hemangioendothelioma. Cancer 1966: 19: 2005-2017. ROBBINS, S. L.: Pathology. W. B. Sawnders Company, Philadelphia 1969, pp. 614-616. SALAMA, N. & HILMY, A: A case of an extensive angioendothelioma of the soft tissues of the lower jaw and the floor of the mouth. Br. Dent. J. 1951: 90: 71-72. SUKLAR, G. & MEYER, I.: Vascular tumors of the mouth and jaws. Oral Surg. 1965: 19: 335-358. SIIERMAN, P. & CALMAN, C. M.: Hernengioendothelioma of the palate, with wide surgical removal and immediate skin graft. Am. J. Surg. 1955: 89: 692-695. SINGH, J., SIDHU, B. S. & KANTA, S.: Hemangioendothelioma of the mandible: report of case. J. Oral Surg. 1977: 35: 673-674. STOUT, A. P.: Hemangio-endothelioma: a tumor of blood vessels featuring vascular endothelial cells. Ann. Surg, 1943: 118: 445--464. SWEITZER, S. E. & WINER, L. H.: Hemangioendothelioma. Arch Dermatol. Syphil. 1936: 34: 997-1007. TOTO, P. D. & LAVlERI, J.: Primary hernangiosarcorna-of the jaw. Oral Surg. 1959: 12: 1459-1463. WEsLEY, R. K., MINTZ, S. M. & WERTlIEIMER, F. W: Primary malignant hemangioendothelioma of, the gingiva. Oral Surg, 1975: 39: 103-112. WINTERS, H. P.: Hemangio-endothelioma. Arch. Chir. Neth. 1969: 21: 153-164. WmIERS, S.: Antio-(perivascular) endothelioma about the jaws. Am. J. Roentgenol1930: 24: 534-539. ZAClIARIADES, N., PAPADAKOU, A., KOUNDOURIS, J., CONSTANTINIDIS, J. & ANGELOPOULOS, A. P.: Primary hernangioendotheliosarcoma of the mandible: review of the literature and report of case. J. Oral Surg. 1980: 38: 288-296.
Address: Nicholas Zachariades 40 Papadiamantopoulou St. 15771 Athens Greece
Maxillary angiosarcoma NICHOLAS ZACHARIADES AND PANAYIOTA ECONOMOPOULOU Oral and Maxillofacial Department, "Apostle Paul's" Accidents Hospital , Kiflssia, Athens, Greece and Department of Oral Pathology, Dental School, National University, Ath ens, Greece
ABSTRACT - A malignant tumor of mesenchym al origin, angiosarcoma, is a rare entity deriving from the endothelium of the blood vessels, that very infrequ ently is encountered in the jaws. 46 such cases have only been reported.
(Accepted for publication Zil March 1985)
Angiosarcoma is a very rare tumor considering the abundance of blood vessels in the human body. It is known to develop as a primary tumor or from irradiated benign hemangioma 1.6.16,21,23,27,31 . It usually affects younger people'. Although the cutaneous type is usually encountered in the face and scalp, the intraosseous type is seldom reported in the jaws l ,6,26,36. Because of its rarity, it is seldom included in difTerential diagnosis and almost never diagnosed clinically. Pyogenic granuloma is the more likely diagnosis. The central type will appear as a radiolucent lesion in radiographic examination, which in some cases may simulate a benign lytic lesion ' ,23,26,31. Even microscopically, it is not always easy to distinguish between the benign and the malignant forms , except for the atypical endothelial cells that characterize the latter, Yet it is still difficull to distinguish between hemangioendotheliosarcorna and hemangiopericytosarcoma, and in more anaplastic forms, between these tumors and leiomyosarcoma or fibrosarco-
ma S,22,24,27,2 8,33,36,39. The tumor spreads by local invasion and metastasizes mainly by the bloodstream. The best treatment consists of wide surgical resection followed by chemotherapy. Prognosis is poor; few patients survive longer than 2 years. Pulmonary metastasis is the main cause of dea th 1.14,21,23,26,28,31,36,39 .
Report of a case A 68-year old woman was admitted to the hospital because of a painless swelling of the left infraorbital region (Fig. I) of 2 months duration. The swelling was associated with infraorbital nerve hypesthesia and could also be palpated . intraorally at the corresponding muccobu ccal fold. No regional lymph nod es were palpable. Radiographic examination (Fig. 2) revealed that a diffuse rad iolucency occupied the area between the upper left central incisor and the canine, also extending towards the premolars. The left lateral incisor had been extr acted and the left central incisor and canine had had root canal therapies performed a month previously. (There was no evidence of caries in the central incisor.) The den-
358
ZACHARIADES AND ECONOMOPOULOU
tal work was carried out because these teeth were painful and the swelling of the -area was considered to be an abscess originating from them. Antibiotics had been prescribed, but the swelling did not subside. Suspecting a malignancy, it was decided to admit the patient for a detailed clinical and laboratory examination, which proved within nonnal limits. 2 days later, under local . anesth esia, a mucoperiosteal nap was raised and a biopsy was performed. The pathologist described sheets of mesenchymal cells that resembled endothelial cells and newly fonned fibroblasts. There were a number of mitoses as well as pleomorphism of cells. Vascular channels were also seen in the specimen (Figs. 3, 4). The diagnosis was angiosarcoma; the patient, however, discharged herself following diagnosis and has not been seen since. Fig. J. Appearance of the patient upon admisWe have tried to locate her and we learned that sion. Note the swelling of the left infraorbital she visited a specialized clinic abroad, where a region. new biopsy was taken, which verified the original
"..'
.. . 4'
'.
.... _ ;. ~
• •
, ,, ~
" ..
'-
Fig. 2. Radiograph of the involved area showing a diffuse radiolucency extending between the upper left central incisor and the premolars of th e same side.
. .
-
Fig . 3. Photomicrograph showing atypical capil-
laries lined with anaplastic endothelial cells. The lumina of most of the capillaries are filled with neoplastic cells. (H.&E. x 160.)
359
MAXILLARY ANGIOSARCOMA 9 8 7 1Il
6
~ 5
:34 3 2
I
I
I
1 ............~~~~~.....~.......~.....~~_ 10 20 30 40 50 60 70
age Fig. 5. Age distribution of the cases presented, when indicated in the reports. Fig. 4. Irregular vascular channels lined with proliferating endothelial cells that ar e pleomorphic and show large hyperchromatic nuclei. (H.&E. x 63.)
dia gnosis of angiosarcoma. (We were able to sec the pathologist's report but the micro scopic slides were not available.) The tumor was cons idered inopera ble and chemotherapy was adm inistered . The patient stayed abroad for approximately 6 months. There was some remi ssion of her symptom s for up to about a year, after which she returned home . At that time she started to develop dyspnea, back pain and head aches. She visited the same clinic abroad, where recurrence of the tumor was verified, associated with wide spread metastasis that involved both lungs, the bra in, the liver and the lumbar spinal column. No further biopsies were taken this time and according to her relatives chemotherapy and irradiation were administered. The patient stayed abroad for a month and was brought back home to die, 15 days later, approximately 2 years after the initial biopsy was taken by us.
Discussion Angiosarcoma is a very rare neoplasm; only
46 cases have so far been reported in the oral cavity and the surrounding tissue sl-l.7-21.2+-26,29-J2,.l-l-39. The age of the patients, when recorded, varied, the youngest being I-day old' and the oldest (current report) 68 (Fig.5). The male/female ratio is almost 1:2 and the mandible and the maxilla are affected with approximately the same frequency. There is no difference between
the right and the left side of the face. The radiographic picture, when published in reports, is always osteolytic. The best treatment appears to consist of wide surgical removal of the tumor regardless of the combination of irradiation. The 2-year survival rate is 50% and the 5-year rate is only 25%. We had the opportunity to encounter another such rare case in OUr Institution".
References I. ALBRUlT, C. R .: Angiosarcoma of the gingiva: report of case. J. Oral Surg, 1970: 28: 913-917. 2. ARENA, S.: Somatic tissue tumors in the head and neck. Trans. Acad. Ophthalmol. Otolarygal. 1962: 15: 100. 3. BERGER, A.: Hemangiosarcoma of the mandible (metastatic?) Ann. Dent. 1942: 1: 15-20. 4. BLAKE, H. , BLAKE, F. S. & PATERSON, N. J.: Angiosarcoma. Oral Surg, 1956: 9: 821-825. 5. BLOOM, W. B. & FAWCETI, D. \V.: Textbook ofhistology, W. B. Sawnders Company, Philadelphia 1969, pp. 358-361. 6. Buxnsxs , W. D. & BRIGIITON, C. T.: Malignant hemangioendothelioma of bone. J . Bone Joint Surg , 1965: 47A: 762-772. 7. CALHOUN, J. J .: Malignant hemangioendothelioma (ang iosarcoma). Oral Surg. 1969: 27: 156-160. 8. CARR, M. W.: Congenital bilateral hem an gioendothelioma. J. Oral Surg, 1948: 6: 341-350. 9. CHEYNE, V.D. & SILBERSTEIN, H. E.: Hemangio-endothelioma. Am. J. Orthod. 1942: 28: 703-721.
360
ZACHARIADES AND ECONOMOPOULOU
10. DELARUE, J.: Observations de tumeurs reticulaires malignes adebut maxillaire en generalisant rapidement. Rev. Stomatal. Chir. Maxillofac. 1957: 58: 603-610. 11. ENDOKIMOV, A. I., GORBUSCIiINA, P. M. & VOROBEZ, I. I.: Malignant hemangioendothelioma of the mandible. Stomatologia 1963: 42: 43--47. 12: FARR, H. W., GARANDANG, C. M. & Huvos, A G.: Malignant vascular tumors of the head and neck. Am. J. Surg. 1970: 120: 501-504. 13. GANDIII, P. K.: Hemangiosarcoma (malignant hemangioendothelioma) of the mandible in a child. Oral Surg. 1966: 22: 359-362. 14. GIRARD, C., WAINE, C. J. & GRAHAM, J. H.: Cutaneous angiosarcoma. Cancer 1970: 23: 868-883. 15. GONDAERT, M.: Angiosarcome du maxillaire superieur (Hemangio-endotheliorne malin). Rev. Stomato-Odontol. 1968: 15: 159-172. 16. HARTMAN, W. H. & STEWART, F. W.: Hemangioendothelioma of bone; unusual tumor characterized by indolent course. Cancer 1962: 15: 846-854. 17. HENNY, F. A.: Angioma of the maxilla in a 3-month old infant: report of case. J. Oral Surg. 1949: 7: 250-252. 18. KRISIINA, G., TIAGI, G. K. & TIAGI, P. S.: Surg, Hemangioendotheliosarcoma. Int. 1968: 50: 377-380. 19. KUPCIIIK, B.:M. & FORLOv, P.: Angiosarcoma of the tongue. Stomatologiia 1971: 50: 91-93. 20. LEGG, Q. J. & FITCH,W M.: Hemangioendothelioma: review of the literature with a report of two cases. South Surg, 1950: 16: 803-811. 21. NALDlcK, R. A.: Angiosarcoma of the mandible. Plast. Reconstr. Surg. 1969: 43: 92-95. 22. ORLlAN, A. I.: Benign hemangiopericytoma of the tongue. J. Oral Surg. 1973: 31: 936-938. 23. OTIS, J.: Hemangioendothelioma of bone. Surg. Gynecol. Obstet. 1969: 127: 295-305. 24. PIIILlPS, H., BROWN, A. & BALL, M.: Hemangioendothelioma: report of case. J. Oral Surg. 1969: 27: 286-288. 25. PINDBORG, J. J. & PIIILlPSEN, H. P.: Malignant angioblastoma of bone. Acta Pathol. Microbial. Scand. 1960: 49: 408--416. 26. QmNN, J. H., MCCONNELL, H. A. & LEONARD, G. L.: Multifocal angiosarcoma of the
27.
28. 29.
30.
31.
32.
33.
34.
35.
36.
37. 38.
39.
gingiva: report of case. J. Oral Surg. 1970: 28: 215-217. RAMSEY, H. J.: Fine structure of hernangiopericytoma and hemangioendothelioma. Cancer 1966: 19: 2005-2017. ROBBINS, S. L.: Pathology. W. B. Sawnders Company, Philadelphia 1969, pp. 614-616. SALAMA, N. & HILMY, A: A case of an extensive angioendothelioma of the soft tissues of the lower jaw and the floor of the mouth. Br. Dent. J. 1951: 90: 71-72. SUKLAR, G. & MEYER, I.: Vascular tumors of the mouth and jaws. Oral Surg. 1965: 19: 335-358. SIIERMAN, P. & CALMAN, C. M.: Hernengioendothelioma of the palate, with wide surgical removal and immediate skin graft. Am. J. Surg. 1955: 89: 692-695. SINGH, J., SIDHU, B. S. & KANTA, S.: Hemangioendothelioma of the mandible: report of case. J. Oral Surg. 1977: 35: 673-674. STOUT, A. P.: Hemangio-endothelioma: a tumor of blood vessels featuring vascular endothelial cells. Ann. Surg, 1943: 118: 445--464. SWEITZER, S. E. & WINER, L. H.: Hemangioendothelioma. Arch Dermatol. Syphil. 1936: 34: 997-1007. TOTO, P. D. & LAVlERI, J.: Primary hernangiosarcorna-of the jaw. Oral Surg. 1959: 12: 1459-1463. WEsLEY, R. K., MINTZ, S. M. & WERTlIEIMER, F. W: Primary malignant hemangioendothelioma of, the gingiva. Oral Surg, 1975: 39: 103-112. WINTERS, H. P.: Hemangio-endothelioma. Arch. Chir. Neth. 1969: 21: 153-164. WmIERS, S.: Antio-(perivascular) endothelioma about the jaws. Am. J. Roentgenol1930: 24: 534-539. ZAClIARIADES, N., PAPADAKOU, A., KOUNDOURIS, J., CONSTANTINIDIS, J. & ANGELOPOULOS, A. P.: Primary hernangioendotheliosarcoma of the mandible: review of the literature and report of case. J. Oral Surg. 1980: 38: 288-296.
Address: Nicholas Zachariades 40 Papadiamantopoulou St. 15771 Athens Greece